Discusses lupus, an autoimmune disorder. Focuses on systemic lupus erythematosus (SLE). Covers symptoms and how it is diagnosed. Covers treatment with medicines like corticosteroids, NSAIDs, and antimalarials. Discusses the importance of good self-care.

Lupus (Systemic Lupus Erythematosus)

Topic Overview

What is systemic lupus erythematosus, or lupus?

Lupus is an autoimmune disease. This means that the body's natural defense system (immune system) attacks healthy tissues instead of attacking only things like bacteria and viruses. This causes inflammation.

Although some
people with lupus have only mild symptoms, the disease is lifelong and can
become severe. But most people can control their symptoms and prevent severe
damage to their organs. They do this by seeing their doctors often for
checkups, getting enough rest and exercise, and taking medicines.

This topic focuses on systemic lupus erythematosus (SLE), the most common
and most serious type of lupus. But there are other types of lupus, such as discoid or cutaneous lupus, drug-induced systemic lupus, and neonatal lupus.

What causes lupus?

The exact cause of lupus is not
known. Experts believe that some people are born with certain
genes that affect how the immune system works. These people are more likely to get lupus. Then a number of other things can trigger
lupus attacks. These include viral infections, including the virus that causes
mononucleosis, and sunlight.

Although these things can trigger
lupus, they may affect one person but not another person.

What are the symptoms?

Lupus symptoms vary
widely, and they come and go. The times when symptoms get worse are called
relapses, or flares. The times when symptoms are under control are called
remissions.

Common symptoms include feeling very tired and having
joint pain or swelling (arthritis), a fever, and a
skin rash. The rash often happens after you have been in the sun. You may have mouth sores
and hair loss. Over time, some people with lupus have problems with
the heart, lungs, kidneys, blood cells, or
nervous system.

How is lupus diagnosed?

There is no single test
for lupus. Because lupus affects different people in different ways, it can be
hard to diagnose.

Your doctor will check for lupus by examining
you, asking you questions about your symptoms and past health, and doing some urine and blood tests.

The doctor may also recommend
other medicines that slow down the immune system (immunosuppressants).

How can you manage lupus?

One of the goals of
controlling mild to moderate lupus symptoms is to prevent flares. You can:

Rest to reduce stress.

Avoid the
sun. Wear sunscreen and protective clothing when you are outside.

Exercise regularly to prevent fatigue and joint stiffness.

Stop
smoking.

Watch your symptoms. If they seem to be getting worse, take steps to control them. For example, if your fatigue, pain, or rash gets worse, it may be a sign of a flare.

With good self-care, most people who have lupus can keep
doing their regular daily activities.

It is important to learn
about lupus so that you can understand how it might affect your life and how
you can best cope with it. Also, help your family and friends understand your
limitations and needs when your symptoms flare. Build a support system of
family, friends, and health professionals.

Cause

The exact cause of lupus is not
known. Experts believe that some people are born with certain
genes that affect how the immune system works. These people are more likely to get lupus. Then a number of other things can trigger
lupus attacks. These include certain medicines, certain chemicals, tobacco smoke, certain infections, and sunlight. Although these things can trigger
lupus, they may affect one person but not another person.

Symptoms

Lupus symptoms depend on what body organs are
affected and how seriously they are affected.

Common symptoms

Many people have these symptoms:

Fatigue: Lupus causes mild to extreme fatigue. Even mild cases of lupus make it hard to do daily activities and exercise. Increased fatigue is a
classic sign that a symptom flare is about to occur.

Joint and muscle pain: Joints may be painful, red, and warm. They may swell. Morning stiffness may also be felt.
Lupus arthritis often occurs on both sides of the body at the same time. It's most often felt in the wrists, the small joints of the hands, and the elbows, knees, and
ankles.

Skin problems: Skin rashes are often an important clue to the
diagnosis. Many people have a
butterfly rash over the cheeks and bridge of the nose. Other common skin symptoms
include skin sores or flaky red spots on the arms, hands, face, neck, or back;
mouth or lip sores; and a scaly, red or purple raised rash on the face, neck,
scalp, ears, arms, and chest.

Sensitivity to light: Exposure to
ultraviolet light (such as sunlight or tanning
parlors) typically makes the skin rash worse and can trigger lupus flares.
Many people with lupus are sensitive to light, with fair-skinned people
tending to be more sensitive.

Fever: People with lupus will
sometimes have a low-grade fever related to the disease. Fever is sometimes a
first sign of the disease.

Changes in weight: People with lupus may lose weight when their disease is active
(flaring).

Swollen glands: Over time, people with lupus may get
swollen
lymph glands during a flare.

Headaches: These are usually related to stress and tension but can be related to a lupus flare. Many people who have lupus get migraine headaches.

Other symptoms

Raynaud's phenomenon: Some people with lupus have Raynaud's phenomenon. It affects the small vessels that supply blood to the skin and the
soft tissues under the skin of the fingers and toes. It causes them to turn white
and/or blue or red. The skin affected will feel numb, tingly, and cold to the
touch.

Hair loss: People with lupus may
have periods of hair loss, either in patches or spread evenly over the
head. This hair loss usually isn't permanent.

Inflammation of blood vessels in the skin (cutaneous
vasculitis): Inflammation or bleeding from the blood
vessels can lead to small or large blue spots or small reddish spots on the
skin or nail beds.

What Happens

The course of lupus varies by
individual and is hard to predict, because symptoms come and go. Lupus usually
develops so slowly that a person may not notice the symptoms for a long
time. Sometimes lupus develops and progresses rapidly.

Periods of time when you have lupus symptoms are called
flares or relapses. Periods of time when your symptoms are under control are called
remissions. Flares and
remissions can occur abruptly, unexpectedly, and without clear cause. There is
no way to predict when a flare will happen, how bad it will be, or how long it
will last. When you have a lupus flare, you may have new symptoms in addition
to those you have had in the past.

Children can get lupus, though
it more commonly develops in the teen years or later. Lupus in children appears
to be more severe than in adults when vital organs, such as the kidneys and
heart, are involved.

Some people with lupus have
complications such as kidney and heart problems. There are also concerns if you have lupus and are pregnant.

Living with lupus

Most people with lupus are able to continue their usual
daily activities. But when your symptoms flare, you may find that you need to cut back on your activity level, get help
with child care, or change the way you work. Or you may find that you need time off from all daily activities.

Most people
with lupus can expect to live a normal or near-normal life span. This depends
on how severe your disease is, whether it affects vital organs (such as the
kidneys), and how severely these organs are affected.

A key to living with lupus is communication. Stay in touch with your doctor about new or increased symptoms, side effects of medicines, and your worries and anxieties. Talk with your family, friends, and employer so they understand what you can and can't do, and what they can do to support you.

Chemical exposure.
Suspected chemical toxins include trichloroethylene in well water and silica
dust. Hair dyes and straighteners, linked to lupus in the past, are no longer
thought to trigger lupus.

What about hormones?

Hormones, including those used for hormone replacement therapy or birth control, don't cause lupus. But they may have some effect on it.

Most women don't have symptom flares during pregnancy, but a few women do when their estrogen levels are high.

Although most women who get lupus are ages 15 to 45, when estrogen levels are higher, a number of women get lupus after menopause, when estrogen levels are low.

The hormones in birth control pills have not proved to be harmful in women who have stable, moderate lupus.1 Women with lupus that isn't well controlled may choose to use nonhormonal birth control methods. These include a copper intrauterine device (IUD), a condom, or a diaphragm. To learn more, see the topic Birth Control.

Talk with your doctor about whether you should use hormonal birth control or hormone replacement.

When To Call a Doctor

Call 911 or other emergency services immediately if you have symptoms of a heart attack. These may include:

Chest pain or pressure, or a strange feeling in the chest.

Sweating.

Shortness of breath.

Nausea or vomiting.

Pain, pressure, or a strange feeling in the back, neck, jaw, or upper belly, or one or both shoulders or arms.

Lightheadedness or sudden weakness.

A fast or irregular heartbeat.

After you call 911, the operator may tell you to chew 1 adult-strength or 2 to 4 low-dose aspirin. Wait for an ambulance. Do not try to drive yourself.

Call 911 or other emergency services immediately if you have one or more of the following
signs of a stroke:

Sudden numbness, tingling, or weakness in
or an inability to move (paralysis) part or all of one side of the body (such
as the face, arm, and leg)

Vision changes that come on suddenly,
such as dimness, blurring, double vision, or loss of vision in one or both
eyes

For more complicated cases of lupus, a rheumatologist is
usually the primary doctor. Other specialists are consulted as needed.

For mental health problems such as
depression, anxiety, psychosis, or other behavioral changes, see your family
medical doctor or internist, or a
psychiatrist.

For the treatment of organ
problems, a doctor who specializes in diseases of that particular organ system
may work together with a rheumatologist or immunologist. The following
practitioners typically treat vital organ problems caused by lupus:

Checking for possible organ damage

Urinalysis to
check for protein and cells, signs of possible kidney
problems.

Kidney biopsy, if your doctor sees
signs of kidney inflammation. This test may help your doctor find the best
treatment for you. Only a small number of people with lupus need a kidney
biopsy.

To look for other possible causes of symptoms, imaging
tests are sometimes done, depending on which organ systems are involved.
Imaging tests include
CT scan,
echocardiogram,
MRI, and
X-rays.

Treatment Overview

Your treatment choices for lupus depend on how severe your symptoms are, whether your organs are affected, and how much your symptoms are affecting your daily life. Your treatment plans should be tailored to your individual needs and will change over time, as the disease flares or ebbs. There currently is no cure for lupus.

Treatment for mild lupus

The goal of treatment for mild
lupus is to prevent symptom flares—when fatigue, joint
pain, and rash get worse.

Get regular checkups with your
doctor, instead of waiting until your disease flares. When flares
do occur, the goal is to treat them right away to limit any damage to body
organs.

Avoid the sun. If you must be in the sun,
cover your arms and legs, wear a hat, and apply broad-spectrum sunscreen
(covering both
UVA and UVB rays) with a high sun protection factor (such as
SPF 50) to protect your skin.

Use
corticosteroid cream for rashes.

Take acetaminophen or nonsteroidal
anti-inflammatory drugs (NSAIDs) and get plenty of rest for
mild joint or muscle pain and fever.

Take
antimalarial medicines, especially for skin rashes. They also help with fatigue and joint and muscle pain.

If you develop serious kidney disease that cannot be controlled with medicine, you may need dialysis or a kidney transplant.

Self-care

Good self-care is essential to managing lupus. A healthy lifestyle may reduce how often you have flares and how severe they are. It can improve your quality of life. Good self-care also helps decrease the risk of heart attack and stroke.

Self-care includes getting regular exercise and eating a healthy diet. To learn more, see Home Treatment.

What to think about

Taking corticosteroids by mouth and
being physically inactive put people with lupus at great risk of bone thinning (osteoporosis). Getting an adequate supply of
calcium and
vitamin D may slow the bone thinning process. Your
doctor may also prescribe bisphosphonates, a type of medicine
that is also used for the prevention and treatment of osteoporosis. To learn more, see the topic
Osteoporosis.

Lupus treatment is complicated by several things.
The course and pattern of lupus symptoms vary widely.
Flares and remissions can occur at any time, making it hard to tell how you are responding to treatment or which treatments are most helpful.
Some treatment side effects can be as troubling as the symptoms of lupus.

It may not be possible to completely eliminate all of
your symptoms for long periods of time, especially without the side effects
from medicines. Work closely with your doctor to reach a balance
between reasonably controlling your symptoms, preventing damage to your organs,
and minimizing side effects of long-term drug treatment. For example, you may
take a dose of medicine that will control lupus enough to prevent organ
damage, but you may still have symptoms such as mild skin rash, muscle aches,
and joint pain.

Using higher doses of medicines for a long time increases the
risk of serious side effects. Your doctor will prescribe a dose
that controls only the most serious, life-threatening symptoms and balances the
risks of the medicines with the benefits of controlling your symptoms.

Prevention

There is currently no way to prevent
lupus. But
people who smoke may be more likely to get lupus.
Avoiding smoking and perhaps other tobacco products may decrease your risk.

Home Treatment

Good self-care is essential to
managing
lupus. Learn to
recognize your body's warning signs of a flare. Warning signs may include
increased fatigue, joint pain, rash, or fever. When you notice any of these
signs, take steps to control your symptoms.

Get plenty of rest. Some people with lupus need
up to 12 hours of sleep every night.

Pace yourself. Limit tiring
activities.

Ask others for help. Don't try to do everything
yourself.

Take short breaks from your usual daily activities.
Consider cutting down on work hours or getting help with parenting
responsibilities, at least during periods when lupus symptoms are
severe.

Exercise regularly. Physical activity boosts energy and
helps you stay in good condition. Walking and swimming are good forms of
exercise for people with lupus.

If you suspect that
depression is contributing to your fatigue, get prompt
treatment from your doctor, a mental health professional, or both.

Taking care of your skin and health

Take care of your skin. Ask your
doctor about the use of
corticosteroid creams to relieve skin symptoms that
are particularly troublesome. If you are bothered by the way a lupus rash looks
on your face or if you have scars from lupus, you can try makeup, such as
Covermark, to cover the rash or scars.

Ultraviolet (UVA and UVB) light triggers disease
flares in most people who have lupus. Exposure to
ultraviolet light, as from sunlight, can trigger or start skin rash, joint
pain, or fatigue, or it can make these symptoms worse. To minimize your
exposure to ultraviolet light:

Avoid the sun. If you must be in the sun, cover
your arms and legs, wear a hat, and apply broad-spectrum sunscreen (covering
both UVA and UVB rays) with a high sun protection factor
(50
SPF or higher) to protect your skin. Reapply sunscreen
after swimming, sweating, or toweling off. Experiment with sunscreens. Some may
irritate your skin or wash off too easily.

Avoid going out when the
sun's rays are the strongest. In most areas, this is between the hours of 10
a.m. and 4 p.m., especially during the summer.

Good general care is essential. A
healthy lifestyle not only improves your quality of life but may also reduce
your chances of having more frequent and severe flares. Taking good general care of yourself also helps decrease the risk of heart attack and stroke. Good care
includes:

Getting vaccinations to help protect you from
illnesses such as pneumonia and the flu. But some vaccinations are not safe if you have lupus. Talk to your doctor about your vaccination schedule.

Building a support
system of family, friends, and health professionals.

If you have lupus and are a woman in your childbearing
years, pay special attention to
pregnancy-related concerns, both before you conceive and
while pregnant. Most women with well-controlled lupus can take birth control
pills if they choose. And for most women, lupus
won't interfere with becoming pregnant or with pregnancy. But some women
with lupus, especially those with active disease, are at higher risk of
problems from pregnancy. All women of childbearing age should check with
their
rheumatologist when they are planning to become
pregnant.

Home treatment and regular checkups are sometimes
enough for managing mild lupus or for periods of
remission. Be sure to have regular checkups. These checks are important to help find
and treat progressive organ damage.

It is important that the
people in your life understand what lupus is, how it affects your life, and how
you can best cope with it. Help them understand your limitations and needs when
your symptoms flare. Support groups are great places to learn coping strategies
from others.

Medications

Medicines can't cure
lupus, but they
can control many symptoms and often can prevent or slow organ damage.

Medicine treatment for lupus
often involves reaching a balance between preventing organ damage, having an acceptable quality of life, and
minimizing side effects. You will need to see your doctor often to see how you're doing and check for medicine side effects.

Your doctor may have to change the dose and
combinations of medicines until you reach the best possible balance.

Medicine choices

If you have mild disease or symptoms that affect your
quality of life but you don't have organ-threatening problems, your doctor may
prescribe:

Acetaminophen or nonsteroidal anti-inflammatory drugs
(NSAIDs), sometimes in combination with antimalarial
drugs. Acetaminophen and NSAIDS are often enough to reduce symptoms.

If you have had blood clots in a vein or
artery (venous or arterial thrombosis), or have
antiphospholipid antibody syndrome, which increases
your risk for blood clots, your doctor may prescribe a blood thinner
(anticoagulant). This is especially important if you
already have blood clots. Aspirin is a nonsteroidal anti-inflammatory drug (NSAID) that is sometimes used to slow blood clotting
in
antiphospholipid antibody syndrome.

What to think about

Some
lupus medicines, like acetaminophen and prednisone, are considered safe
during pregnancy. Others may not be. You may not be able to stop taking lupus
medicines after becoming pregnant. Or you may need to start taking medicines
for a symptom flare. If possible, talk to your doctor
before becoming pregnant so you can learn about the effect lupus may
have on your pregnancy.

Because corticosteroids are powerful medicines and can
cause serious side effects, your doctor will recommend the lowest dose that will
give the most benefit.

Some people with lupus are sensitive to antibiotic medicines
called sulfonamides (sulfa medicines). These include Bactrim, Septra, and many
others. Your doctor can prescribe medicines that don't contain sulfa, if
needed.

People with lupus can go
into spontaneous
remission. If this happens to you,
your doctor may cut back your medicine over time or stop your medicine.

Surgery

Surgery isn't used to treat mild or moderate
symptoms of
lupus. But it
may be considered for people who have permanent, life-threatening
kidney damage. A kidney transplant or kidney
dialysis may be done instead of continuing long-term
treatment with high doses of medicines that have serious side effects.

Other Treatment

While some people with
lupus try
alternative or complementary therapies (such as special diets, fish oils, or
chiropractic treatment), these are not proven treatments for lupus.

Some therapies that focus on relaxation can help you cope with having a
long-lasting (chronic) disease and may significantly improve your quality of
life. These relaxation therapies include yoga,
guided imagery, and massage.

Experimental therapies for lupus

Medicines and
therapies now being studied are meant to change how the
immune system works so that they can keep the disease
from progressing. These newer treatments include stem cell transplantation and
biologic treatment.

Immunoablation with or without stem cell transplantation is
being studied as a treatment for severe lupus that has not been controlled with all other treatments. Immunoablation uses powerful
drugs to wipe out the damaged immune system. After immunoablation, either the
bone marrow is allowed to replace itself, or it is partially replaced through a
stem cell transplant. The transplant replaces damaged or destroyed bone marrow
cells with healthy cells, or stem cells. Stem cells are immature cells that are
produced in the bone marrow. They can divide to produce more stem cells. Or they can
mature into red blood cells, white blood cells, and platelets. More study of
these treatments for lupus is needed.

Biologic treatment blocks specific steps in the
lupus
autoimmune process without suppressing the entire
immune system. Researchers are currently experimenting with very specific
substances, such as antibodies and nucleotides, that block certain steps
of the autoimmune process. Rituximab is an antibody directed against
certain immune cells that may have a role in lupus. It is approved for treating
rheumatoid arthritis. Studies are looking at the use of rituximab for lupus. It
is beginning to be used for lupus flares that have not responded to other
immunosuppressive therapies. In some cases, rituximab has been associated with
serious side effects such as breathing difficulty, heart problems, or severe
infection. So the use of rituximab is closely watched.

DHEA (also
called prasterone in the United States) is an androgenic dietary supplement
that is derived from the wild yam. Experts suggest only using
pharmaceutical-grade (versus "natural") DHEA. Results of research are mixed. But most studies show the drug has no more impact on the lupus itself than a placebo.2 The most common side effects of DHEA are acne and facial hair
growth in women and hair loss in men. Because this supplement is a hormonal
substance, talk to your doctor before using it. And have your DHEA blood levels
checked every 6 months. Long-term effects are not known.

Plasmapheresis, the removal of some parts
of the blood, is rarely used as treatment for lupus except for certain severe cases in
which other treatments have not worked.3

Intravenous gamma globulin (IgG) may be used to treat lupus that has not responded to other treatment. There is not good evidence that this works for lupus.

Other Places To Get Help

Organizations

Lupus Research Institute (U.S.)

Web Address:

www.lupusresearchinstitute.org

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases (U.S.)

Other Works Consulted

Bertsias GK, et al. (2007). EULAR recommendations for
the management of systemic lupus erythematosus (SLE) report of a task force of
the European Standing Committee for International Clinical Studies Including
Therapeutics (ESCISIT). Annals of the Rheumatic Diseases, 67(2): 195–205.

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